Department of Neurology, Addis Ababa University, Addis Ababa, Ethiopia.
J Neurol Sci. 2010 Mar 15;290(1-2):142-5. doi: 10.1016/j.jns.2009.12.009. Epub 2009 Dec 31.
Cervical spondylotic amyotrophy (CSA) is considered a syndrome of (1) unilateral upper extremity weakness and atrophy, (2) affecting either the proximal or distal musculature, (3) without sensory impairment or lower extremity dysfunction.
The authors report a novel case of bilaterally symmetric CSA with blurring of the proximal-distal distinction, discuss the pathophysiology, and review the literature.
A 45 year old man presented with a several year history of insidiously progressive bilaterally symmetric upper extremity weakness and wasting, profound in the proximal musculature and moderate to severe in the distal muscle groups.
Based on the clinical, neuroimaging and electrodiagnostic features, this patient harbors a more severe phenotype of the classical syndrome.
The authors propose expanding the generally accepted definition of CSA to include this bilaterally symmetric form of disease, thereby minimizing diagnostic error or delay. Additionally, based on this case and a review of the literature, adherence to the proximal-distal distinction should be avoided since it is commonly blurred. Accurate diagnosis is crucial since this presentation mimics the motor neuron disease variant Vulpian-Bernhardt syndrome. The importance of early diagnosis is underscored by reports of significant improvement with timely surgical decompression.
颈源性肌萎缩症（CSA）被认为是一种（1）单侧上肢无力和萎缩的综合征，（2）影响近端或远端肌肉，（3）无感觉障碍或下肢功能障碍。
作者报告了一例新的双侧对称 CSA 病例，近端-远端界限模糊，并讨论了其病理生理学，并复习了文献。
一名 45 岁男性出现数年来进行性双侧对称上肢无力和消瘦，近端肌肉明显，远端肌肉中度至重度。
根据临床、神经影像学和电诊断特征，该患者具有更严重的经典综合征表型。
作者建议扩大 CSA 的一般定义，包括这种双侧对称形式的疾病，从而最大限度地减少诊断错误或延迟。此外，基于该病例和文献复习，应避免遵循近端-远端的区别，因为它通常是模糊的。准确的诊断很重要，因为这种表现类似于运动神经元病变异型 Vulpian-Bernhardt 综合征。早期诊断的重要性在于及时手术减压可显著改善病情。
